ELECTRICAL STATUS EPILEPTICUS IN SLEEP

Course overview

ELECTRICAL STATUS EPILEPTICUS IN SLEEP (ESES), A CHILDHOOD-ONSET EPILEPTIC ENCEPHALOPATHY, IS CHARACTERIZED BY EPILEPSY, COGNITIVE REGRESSION, AND MARKED ACTIVATION OF EPILEPTIFORM ACTIVITY DURING NON-RAPID EYE MOVEMENT (NREM) SLEEP TO PRODUCE AN ELECTROENCEPHALOGRAPHY (EEG) PATTERN OF NEAR-CONTINUOUS SPIKE-WAVE DISCHARGES. DIFFERENT ETIOLOGIES, BOTH WITH OR WITHOUT STRUCTURAL ABNORMALITIES OF THE BRAIN, HAVE BEEN ASSOCIATED WITH ESES. TYPICALLY CHILDREN, BETWEEN 2 AND 12 YEARS OF AGE WITH A PEAK BETWEEN 3 AND 5 YEARS, PRESENT WITH INFREQUENT SEIZURES (FOCAL AND/OR GENERALIZED) AND STAGNATION OR REGRESSION OF THE DEVELOPMENT. THE EXISTING TREATMENT PARADIGM IS BASED ON UNCONTROLLED STUDIES AND CASE SERIES AND PRIMARILY FOCUSED USE OF TRADITIONAL ANTIEPILEPTIC DRUGS (AEDS), INCLUDING BENZODIAZEPINES, CORTICOSTEROIDS, EPILEPSY SURGERY, AND OTHER NON-PHARMACOLOGIC THERAPIES(IVIG, THE KETOGENIC DIET. THIS ACTIVITY REVIEWS THE EVALUATION AND MANAGEMENT OF ESES AND HIGHLIGHTS THE ROLE OF THE HEALTHCARE TEAM IN EVALUATING AND TREATING PATIENTS WITH THIS CONDITION.